Childhood-onset systemic lupus erythematosus (cSLE) and malignancy: a nationwide multicentre series review

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article
Data de publicação
2024
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Scopus
Web of Science
PubMed
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BMC
Autores
BRUFATTO, Matheus Zanata
LANCAS, Sean Hideo Shirata
FERNANDES, Taciana de Albuquerque Pedrosa
SAKAMOTO, Ana Paula
TERRERI, Maria Teresa
SZTAJNBOK, Flavio Roberto
BICA, Blanca Elena Rios Gomes
FERRIANI, Virginia Paes Leme
Citação
ADVANCES IN RHEUMATOLOGY, v.64, n.1, article ID 13, 7p, 2024
Projetos de Pesquisa
Unidades Organizacionais
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Resumo
BackgroundIncreased malignancy frequency is well documented in adult-systemic lupus erythematosus (SLE), but with limited reports in childhood-onset SLE (cSLE) series. We explored the frequency of malignancy associated with cSLE, describing clinical and demographic characteristics, disease activity and cumulative damage, by the time of malignancy diagnosis.MethodA retrospective case-notes review, in a nationwide cohort from 27 Pediatric Rheumatology centres, with descriptive biopsy-proven malignancy, disease activity/damage accrual, and immunosuppressive treatment were compiled in each participating centre, using a standard protocol.ResultsOf the 1757 cSLE cases in the updated cohort, 12 (0.7%) developed malignancy with median time 10 years after cSLE diagnosis. There were 91% females, median age at cSLE diagnosis 12 years, median age at malignancy diagnosis 23 years. Of all diagnosed malignancies, 11 were single-site, and a single case with concomitant multiple sites; four had haematological (0.22%) and 8 solid malignancy (0.45%). Median (min-max) SLEDAI-2 K scores were 9 (0-38), median (min-max) SLICC/ACR-DI (SDI) score were 1 (1-5) Histopathology defined 1 Hodgkin's lymphoma, 2 non-Hodgkin's lymphoma, 1 acute lymphoblastic leukaemia; 4 gastrointestinal carcinoma, 1 squamous cell carcinoma of the tongue and 1 anal carcinoma; 1 had sigmoid adenocarcinoma and 1 stomach carcinoid; 3 had genital malignancy, being 1 vulvae, 1 cervix and 1 vulvae and cervix carcinomas; 1 had central nervous system oligodendroglioma; and 1 testicle germ cell teratoma.ConclusionEstimated malignancy frequency of 0.7% was reported during cSLE follow up in a multicentric series. Median disease activity and cumulative damage scores, by the time of malignancy diagnoses, were high; considering that reported in adult series.
Palavras-chave
Childhood-onset systemic lupus erithematosus, Registry, Malignancy, Leukaemia, Lymphoma, Solid malignancy
URI
https://observatorio.fm.usp.br/handle/OPI/58949
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Coleções
Artigos e Materiais de Revistas Científicas - FM/MPE